Enhanced Notch3 signaling contributes to pulmonary emphysema in a Murine Model of Marfan syndrome

Marfan syndrome (MFS) is a heritable disorder of connective tissue, caused by mutations in the fibrillin-1 gene. Pulmonary functional abnormalities, such as emphysema and restrictive lung diseases, are frequently observed in patients with MFS. However, the pathogenesis and molecular mechanism of pul...

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Veröffentlicht in:	Scientific reports 2020-07, Vol.10 (1), p.10949-10949, Article 10949
Hauptverfasser:	Jespersen, Kathryn, Liu, Zhibo, Li, Chenxin, Harding, Paul, Sestak, Kylie, Batra, Rishi, Stephenson, Christopher A., Foley, Ryan T., Greene, Harrison, Meisinger, Trevor, Baxter, B. Timothy, Xiong, Wanfen
Format:	Artikel
Sprache:	eng
Schlagworte:	631/136 692/699/1785 Alveoli Animal models Animals Apoptosis Connective tissue diseases Disease Models, Animal Emphysema Epithelium Female Fibrillin Humanities and Social Sciences Lung diseases Lungs Male Marfan syndrome Marfan Syndrome - complications Mice Mice, Inbred C57BL multidisciplinary Pulmonary Emphysema - etiology Pulmonary Emphysema - metabolism Pulmonary Emphysema - pathology Receptor, Notch3 - genetics Receptor, Notch3 - metabolism Science Science (multidisciplinary) Secretase Septation
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